Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.celrep.2021.108795 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
The Role of Gene Expression Dysregulation in the Pathogenesis of Mucopolysaccharidosis: A Comparative Analysis of Shared and Specific Molecular Markers in Neuronopathic and Non-Neuronopathic Types of the Disease.
Int J Mol Sci
December 2024
Department of Molecular Biology, Faculty of Biology, University of Gdansk, Wita Stwosza 59, 80-308 Gdansk, Poland.
Mucopolysaccharidosis (MPS) comprises a group of inherited metabolic diseases. Each MPS type is caused by a deficiency in the activity of one kind of enzymes involved in glycosaminoglycan (GAG) degradation, resulting from the presence of pathogenic variant(s) of the corresponding gene. All types/subtypes of MPS, which are classified on the basis of all kinds of defective enzymes and accumulated GAG(s), are severe diseases.
View Article and Find Full Text PDFShared Gene Expression Dysregulation Across Subtypes of Sanfilippo and Morquio Diseases: The Role of PFN1 in Regulating Glycosaminoglycan Levels.
Front Biosci (Landmark Ed)
December 2024
Department of Molecular Biology, Faculty of Biology, University of Gdansk, 80-308 Gdansk, Poland.
Background: Mucopolysaccharidosis (MPS) is a class of hereditary metabolic diseases that demonstrate itself by accumulating incompletely degraded glycosaminoglycans (GAGs). MPS are classified according to the kind(s) of stored GAG(s) and specific genetic/enzymatic defects. Despite the accumulation of the same type of GAG, two MPS diseases, Sanfilippo (MPS III) and Morquio (MPS IV), are further distinguished into subclasses based on different enzymes that are deficient.
View Article and Find Full Text PDFNovel Insights on the Biology and Immunological Effects of Histamine: A Road Map for Allergists and Mast Cell Biologists.
J Allergy Clin Immunol
December 2024
Institute of Allergology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany. Electronic address:
Histamine (CHN, molecular weight 111.15 g/mol) is a well-studied endogenous biogenic amine composed of an imidazole ring attached to an ethylamine side chain. It has a limited half-life of a few minutes within tissues and in circulation.
View Article and Find Full Text PDFMass Spectrometry-Based Glycomics and Proteomics Profiling of On-Slide Digested Tissue from Complex Biological Samples.
Methods Mol Biol
December 2024
Department of Biochemistry, Center for Biomedical Mass Spectrometry, Boston University School of Medicine, Boston, MA, USA.
Mass spectrometry-based investigation of the heterogeneous glycoproteome from complex biological specimens is a robust approach to mapping the structure, function, and dynamics of the glycome and proteome. Sampling whole wet tissues often provides a large amount of starting material; however, there is a reasonable variability in tissue handling prior to downstream processing steps, and it is difficult to capture all the different biomolecules from a specific region. The on-slide tissue digestion approach, outlined in this protocol chapter, is a simple and cost-effective method that allows comprehensive mapping of the glycoproteome from a single spot of tissue of 1 mm or greater diameter.
View Article and Find Full Text PDFValidation of a Simple HPLC/UV Method for Assay and In Vitro Release of Glycosaminoglycan from Pharmaceutical Formulations.
ACS Omega
December 2024
Faculty of Pharmacy, Department of Pharmaceutical Technology, Ege University, Bornova, Izmir 35040, Turkey.
This study encompasses the validation of a simple, rapid, and sensitive HPLC/UV method developed in accordance with the guidelines set by ICH Q2(R2) for obtaining the active pharmaceutical ingredient from the glycosaminoglycan family in topical formulations. Previous methods reported for analyzing glycosaminoglycans in semisolid formulations are relatively complex and time-consuming, involving extraction, purification, and derivatization. This developed analytical method allows for straightforward extraction of the active pharmaceutical ingredient from the matrix, enabling the direct injection of samples.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!